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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.127, No.8, August 2023
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Original Article
Title
Reduction in Blood Pressure with Intravenous Acetaminophen in Pediatric Patients
Author
Norioki Ono Kosho Higuchi Reiko Tsuda Yoko Murakami Mizuka Miki and Naoto Fujita
Department of Pediatrics Hiroshima Red Cross Hospital & Atomic-bomb Survivors Hospital
Abstract
Introduction: Intravenous acetaminophen may be associated with a reduction in blood pressure in adult patients, but there have been few reports in pediatric patients.
Subjects and methods: The retrospective subjects were pediatric patients aged younger than 18 years who were hospitalized between July 2020 and October 2021. Temperature, heart rate, and blood pressure were measured prior to and 15 to 30 minutes after the administration of intravenous acetaminophen. A decrease in the rate of change in blood pressure by ≥ 15% after administration was defined as a reduction in blood pressure.
Results: Intravenous acetaminophen was administered 116 times in 24 patients. Temperature, heart rate, mean blood pressure, systolic blood pressure, and diastolic blood pressure were decreased after administration compared with those before administration. A ≥ 15% reduction in the rate of change was found in 19.0% of patients for mean blood pressure, 6.9% for systolic blood pressure, and 31.0% for diastolic blood pressure. There were no cases of hypotension requiring intervention.
Discussion: Diastolic blood pressure is more likely to decrease than systolic blood pressure with intravenous acetaminophen, and the mechanism of peripheral vasodilation might be involved. Therefore, patients with low circulating blood volume need to be carefully monitored.
Conclusion: Intravenous acetaminophen appears to be a safe drug, but it can cause a reduction in blood pressure in pediatric patients.
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Case Report
Title
A Case of Complete DiGeorge/CHARGE Syndrome in a Newborn Diagnosed Early by Using T-cell Receptor Excision Circles Screening
Author
Satoru Takashima Kei Inomata Shouta Fujito Madoka Nishimura Daisuke Katayama Rina Ootsuka Hidetaka Yoshimatsu and Takeshi Inoue
Department of Neonatology, Kumamoto City Hospital Perinatal Center
Abstract
A girl was born at 37 weeks and 6 days of gestation, weighing 2,346 g. She had multiple congenital malformations including microphthalmia, cleft lip and palate, and auricular hypoplasia. Other complications, such as patent ductus arteriosus, an atrial septal defect, retinal coloboma, and posterior nasal dysplasia, were observed. Based on these abnormalities, and finding the CHD7 variant in genetic testing, she was diagnosed with CHARGE syndrome. She had no amplification of T cell receptor excision circles (TREC) using a pilot screening of newborns for severe combined immunodeficiency disease (SCID). CD3-positive T cells were 8/μL in her lymphocyte subsets. Based on the presence of congenital heart disease, hypoparathyroidism, thymic aplasia, and markedly decreased T cells, she was diagnosed with complete DiGeorge/CHARGE syndrome. She was started on a strict infection prevention protocol from 1 month after birth, and was given hematopoietic cell transplantation (HCT) at the age of 70 days. A newborn TREC screening for SCID was useful in the diagnosis of complete DiGeorge/CHARGE syndrome. Early diagnosis is important as the outcome of HSCT after infection is poor.
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Case Report
Title
A Quadruplet Case of Left Ventricular Non-Compaction Complicated with an Atrial Septal Defect, and Which Showed a Novel ACTC1 Gene Mutation
Author
Yoshikazu Otsubo1) Nobuhiro Ishibashi1) Mari Yokokawa1) and Keiichi Hirono2)
1)Department of Pediatrics, Sasebo City General Hospital
2)Department of Pediatrics, Toyama University
Abstract
Left ventricular non-compaction (LVNC), morphologically characterized by excessive trabeculation and deep recesses of the ventricular wall, is classified by the WHO classification as an unclassified type of cardiomyopathy. Despite accounting for approximately 9.5% of cardiomyopathy in children, the clinical features are extremely diverse, ranging from asymptomatic to severe cardiac dysfunction and some which even require cardiac transplantation.
LVNC is known to be associated with congenital heart disease, we herein report a quadruplet case of LVNC complicated with an atrial septal defect (ASD). Genetic testing confirmed heterozygous missense mutations (c.496C > T, p. Pro166Ser) in the actin, α-cardiac muscle (ACTC1) gene. Born at 35 weeks of gestation, 3 out of 4 had ASD, and all 4, including 1 without ASD, had electrocardiogram (ECG) abnormalities (ventricular hypertrophy, QT prolongation) that did not match ASD, leading to a diagnosis of LVNC based on the echocardiographic findings. An intra-family search revealed that the mother and sister also had ECG abnormalities and LVNC findings, and the same genetic mutation was observed.
Our research showed that the penetration rate and phenotype were not uniform for some cases in which ASD was not complicated despite the presence of an identical ACTC1 gene mutation. The ACTC1 gene is also known as a cardiomyopathy-related gene. This novel mutation combination of ASD and LVNC may have an effect on actin kinetics and cardiac morphogenesis, therefore the ACTC1 gene is considered to be important in predicting the onset of ASD and cardiomyopathy.
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Case Report
Title
An Infant with Diaphanospondylodysostosis Who Has Transitioned to Home Medical Care and Developmentally Progressed until Age Four
Author
Aoi Sugawara1)2) Hiro Nakao1)2) Tomoo Nakamura2) Osamu Miyazaki3) Nozomi Hiraishi4) Kazuhiro Iwama4)5) Mitsuru Kubota2) and Akira Ishiguro1)
1)Center for Postgraduate Education and Training, National Center for Child Health and Development (NCCHD)
2)Department of General Pediatrics & Interdisciplinary Medicine, NCCHD
3)Department of Diagnostic Radiology, NCCHD
4)Department of Pediatrics, Yokohama City University (YCU)
5)Department of Human Genetics, Graduate School of Medicine, YCU
Abstract
Diaphanospondylodysostosis (DSD) is a rare hereditary disorder caused by recessively inherited mutations of the BMPER gene (OMIM *608699) on the short arm of human chromosome 7. Although DSD is categorized as one of the skeletal disorders with a high perinatal mortality rate, we encountered a girl affected by DSD who survived to 4 years and 5 months of age. Although bell-shaped thorax and respiratory failure were present from birth, her respiratory condition was stable after tracheostomy and mechanical ventilation. She was discharged home with mechanical equipment. Although expressive language was slightly delayed, she later developed satisfactory receptive language and could pull herself up. Her genotype was found to be compound heterozygous with one variant being a nonsense mutation (c.1672C>T, p.Arg558Ter), same as the previously reported causative variant of ischiospinal dysostosis (ISD), and with one allele lacking exons 1-3. However, the phenotype in our case was more severe than that of previously reported ISD cases. DSD and ISD may be a spectrum within the same disease entity, and both are BMPER-related skeletal disorders. Outcomes may be improved by medical intervention tailored in accordance with the actual phenotype. Although this disease has been categorized as a skeletal disorder with a high perinatal mortality rate, it has been reported that some patients can grow beyond infancy, as in our case. Further case accumulation is required.
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Case Report
Title
Accidental Ingestion of a Heated Cigarette with an Integrated Metal Piece: A Case Report and Review of Similar Cases
Author
Miki Yamada Taichiro Muto Yosuke Nishio Shoko Ozeki Shuta Kito Naozumi Fujishiro Hirohide Tokunaga and Shinji Hasegawa
Department of Pediatrics, Nagoya Memorial Hospital
Abstract
A heated tobacco cigarette with an integrated metal strip was marketed in September 2021. In this product, a tobacco leaf containing up to 6 mg of nicotine fits into a 12-mm filling, and a 4-mm wide, 0.06-mm thin piece of metal is embedded in the same area.
This report describes a case of accidental ingestion of a heated cigarette with an integrated metal fragment and reviews similar cases from Injury Alert.
A 2-year-old boy presented to our hospital with the chief complaint of nausea 30 minutes after accidentally ingesting a heated tobacco cigarette containing a metal piece. He was fully conscious, and there was no pupillary discrepancy or constriction. An X-ray confirmed the presence of a metal fragment in his stomach. The patient was observed after gastric lavage and administration of activated charcoal. The metal fragment was assumed to have been excreted via defecation within 8 days.
The risks associated with accidentally ingesting a heated cigarette with a built-in metal piece are related to the device's shape, including its short tobacco leaf filling, which makes it easy to ingest. The health risks include the high nicotine content and the possibility of damage to the gastrointestinal tract by the thin, sharp metal fragments, which may be difficult to visualize on X-ray evaluation. Injury Alert has reported similar cases. With the increase in such accidents, appropriate treatment should be considered. Pediatricians and caregivers should be aware of the potential accidental ingestion of heated cigarettes containing a metal fragment and the associated risks.
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